A fifteen-week-old intact female Boston Terrier puppy presented with a seven week history of hyporexia, dysphagia, difficulty ambulating, failure to gain weight, lethargy, hypothermia, and lymphadenomegaly. Physical exam revealed dull mentation, poor body condition, non-ambulatory tetraparesis, severe joint laxity, and bilateral corneal opacities. Imaging showed evidence of cerebral atrophy, hydrocephalus, severe vertebral and interve1iebral disc malformation, epiphyseal dysplasia, and skull malformations. Cytology of blood, cerebrospinal fluid, and joint fluid revealed cytoplasmic vacuolation and cytoplasmic granules throughout. A metabolic panel revealed a positive MPS spot test. All of these findings were consistent with mucopolysaccharidosis. The puppy was euthanized and a necropsy was performed.